Chapter 22. DNA-Repair Deficiency and Cancer: Lessons from Lymphoma

  1. Prof. Dr. Heike Allgayer PhD4,
  2. Prof. Dr. Helga Rehder5 and
  3. Prof. Dr. Simone Fulda6
  1. Krystyna H. Chrzanowska1,
  2. Martin Digweed2,
  3. Karl Sperling2 and
  4. Eva Seemanova3

Published Online: 21 AUG 2009

DOI: 10.1002/9783527627523.ch22

Hereditary Tumors: From Genes to Clinical Consequences

Hereditary Tumors: From Genes to Clinical Consequences

How to Cite

Chrzanowska, K. H., Digweed, M., Sperling, K. and Seemanova, E. (2008) DNA-Repair Deficiency and Cancer: Lessons from Lymphoma, in Hereditary Tumors: From Genes to Clinical Consequences (eds H. Allgayer, H. Rehder and S. Fulda), Wiley-VCH Verlag GmbH & Co. KGaA, Weinheim, Germany. doi: 10.1002/9783527627523.ch22

Editor Information

  1. 4

    University of Heidelberg and DKFZ (German Cancer Research Center) Heidelberg, Medical Faculty Mannheim, Chair of Experimental Surgery, Theodor-Kutzer-Ufer 1–3, 68167 Mannheim, Germany

  2. 5

    Medical University Vienna, Department of Medical Genetics, Währinger Strasse 10, 1090 Wien, Austria

  3. 6

    Ulm University Children's Hospital, Eythstrasse 24, 89075 Ulm, Germany

Author Information

  1. 1

    The Children's Memorial Health Institute, Department of Medical Genetics, Al. Dzieci Polskich 20, 04-736 Warsaw, Poland

  2. 2

    Charité-Universitätsmedizin Berlin, Institut für Humangenetik, Augustenburger Platz 1, 13353 Berlin, Germany

  3. 3

    Charles University Hospital, Department of Clinical Genetics, Vúvalu 84, 15018 Praha, Czechia, Czech Republic

Publication History

  1. Published Online: 21 AUG 2009
  2. Published Print: 17 DEC 2008

ISBN Information

Print ISBN: 9783527320288

Online ISBN: 9783527627523

SEARCH

Keywords:

  • Ataxia-telangiectasia;
  • double-strand breaks;
  • MRN complex;
  • Nijmegen breakage syndrome;
  • T-cell receptor

Summary

A peculiarity of the lymphatic system is its high rate of somatic recombination with associated hypermutability. In this process, DNA double-strand breaks (DSB) are generated and processed, whereby the genes responsible are often also involved in general DSB repair. Germline mutations in these genes are responsible for a particularly high risk for lymphoma. Genetic disorders such as ataxia-telangiectasia (A-T) and Nijmegen Breakage Syndrome (NBS), together with the characteristic somatic mutations discovered in sporadic lymphoma, have made major contributions to the understanding of DNA repair defects and carcinogenesis in general.